Laboratory Data

Biopsy from abdominal lesion showed numerous granulomata composed of epithelioid histiocytes, lymphocytes and multinucleated giant cells, Langhan’s type. Special stains for Acid fast bacilli (ZN, Wade fite) and fungal bodies (PAS) were negative. No abnormal deposits of eosinophilic amorphous material seen. No foreign body seen by polarised light.

( see below for images posted on June 24, 2007)

Sarcoidosis?

Differential Diagnoses: Nodular amyloidosis, Scleromyxoedema, Morphoea, xanthogranuloma

For diagnostic and therapeutic suggestions

Sunil Dogra MD, Associate Professor of Dermatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India on June 17, 2007

An interesting case indeed. I would consider clinical conditions - CTCL, Sarcoidosis and Histiocytosis.

David Elpern MD, Consultant Dermatologist, Williamstown, MA, USA on June 17, 2007

This is a unique case. Do you actually see sarcoid in Malaysia? If so, perhaps this is it. Her case may be "an experiment of one." I'd recommend showing some photomicrographs to some of our dermpaths and then considering therapy. Here's a recent review from the JAAD (which I think you receive) Ted Rosen (one of the authors) would comment, I am sure. Please keep us posted. There are some articles on TNF drugs for sarcoid, but I haven't reviewed them.

This is a very unusual case. There have been some cases of CTCL which presented with a sarcoidal-like pathology. Please see the references affixed. Immunostaining may be of value.

Reference:

Badgwell C, Rosen T.J. Cutaneous sarcoidosis therapy updated. Am. Acad. Dermatol. 2007 Jan;56(1):69-83.

The widely accepted standard therapy for cutaneous sarcoidosis includes
corticosteroids, antimalarials, and methotrexate. However, a better understanding
of the basic immunopathogenic properties of sarcoidosis has elucidated a number
of steps critical to the persistence and progression of disease that may be
vulnerable to treatment by targeted therapy. This article reviews both standard
and newer therapeutic options for cutaneous sarcoidosis.

Omid Zalgari MD, Rasht, Iran on June 17, 2007

My first diagnosis for this very interesting case would be a variant of CTCL, most probably a granulomatous MF and perhaps an unusual form of disseminated granulomatous slack skin. Many thanks for sharing this case.

Ian McColl FRACP, Consultant Dermatologist, John Flynn Medical Centre, Tugun, Queensland, Australia on June 17, 2007

Clinically you would more favour a T cell lymphoma but sarcoidosis can clinically mimic about anything. Was the ACE level raised? You can get a granulomatous T cell lymphoma but is ACE raised then as well?

Rick Sontheimer MD, Professor of Dermatology, Department of Dermatology, University of Oklahoma Health Science Centre, Oklahoma City, OK, USA on June 18, 2007

Agree with CTCL of granulomatous MF subtype.

Bushan Kumar MD, Former Professor of Dermatology, PGIMER, Chandigarh, India on June 18, 2007

I would consider granulomatus MF as the only possibility. Assessment about involvement of other organ system should be made to decide about the therapeutic approach.

Abir Saraswat MD, Consultant Dermatologist, Lucknow, India on June 18, 2007

Granulomatous CTCL is a strong possibility, but it is unlikely that the histopathology would be described as "multiple granulomata with giant cells." It should be more like band-like infiltrate of lymphocytes (atypical or not) admixed with giant cells and some histiocytes. Some of the photographs are strongly reminiscent of a patient with ichthyotic sarcoidosis that I saw some years back.

A histopath review should clear up the issue; perhaps Dr Choon could upload some photomicrographs. A very interesting case worth getting to the bottom of.

Shahbaz Janjua MD, Consultant Dermatologist, Ayza Skin & Research Center, Lalamusa, Pakistan on June 18, 2007

This is a great case. I would consider MF, sarcoidosis, amyloidosis, lepromatous leprosy, disseminated leishmaniasis, atypical mycobacteriosis, and deep fungal infection on clinical grounds.

Khalifa Sharquie MD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on June 19, 2007

Well, it is a very interesting case. The overlying skin changes are poikiloderma-like which are unusual to be seen in sarcoidosis. And sarcoidosis is very rare in Asian people. Granulomatous reaction could be seen in mycosis fungoides. Accordingly the whole picture is suggestive of lymphoma. So please do Kveim test to excude sarcoidosis and do full investigations to confirm CTCL.

Sam Moshella MD, Clinical Professor of Dermatology, Emeritus, Harvard Medical School, Boston, USA on June 20, 2007

I believe the patient has granulomatous MF. It would be interesting to know whether elastic tissue stains were done to support the diagnosis of Slack skin MF.

Choon Siew Eng FRCP, Senior Consultant Dermatologist, Johor Bahru, Malaysia on June 21, 2007

Update on the case. Thank you for your contributions.

We are repeating the full panel of immunostains for lymphoma, CD1a and elastic stain. CT scan from base of skull to pelvis also did not reveal any LN or organomegaly. Will get the histology slides up for VGRD soon. Will keep you all informed of results.

Update on HPE on June 24, 2007

Biopsy from abdominal lesion showed numerous granulomata composed of epithelioid histiocytes, lymphocytes and multinucleated giant cells, Langhan’s type. Special stains for Acid fast bacilli (ZN, Wade fite) and fungal bodies (PAS) were negative. No abnormal deposits of eosinophilic amorphous material seen. No foreign body seen by polarised light. (slide courtesy of Dr Ghazali)

Andrew Carlson MD, Professor, Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, NY, USA on June 2007

Fascinating case.

Predominately intertriginous involvement that is slightly pendulous and erythematous associated with patches of ichthyosis (acquired ichthyosis due lymphoma or sarcoidosis?). The histology can be best classified as diffuse interstitial granulomatous dermatitis with numerous multinucleated giant cells. The associated lymphocytes are small, but hyperchromatic. I don't see any regions of epidermotropism. Overall, I am most suspicious for "granulomatous slack skin syndrome" a variant of mycosis fungoides. The differential diagnosis is sarcoidosis; sarcoid should be diagnosed only after you have excluded lymphoma (look for gene rearrangement and/or abnormal immunophenotype) and infection (mycobacterial
infection, deep fungus, leishmaniasis). What does her CXR show? Elevated ACE or Calcium level?

Thanks for sharing the histology with me.

Update on the case by Choon Siew Eng FRCP on July 20.

Thank you for your contributions. Confirmed GSSD. Her bone marrow and blood film normal and my haem colleague has discharged her back to me. I am thinking of putting her on interferon first like my other patients before resorting to CHOP. There are 42 cases reported. Mainly caucasians and male. Any one with experience with pentostatin? I would appreciate treatment advice